Behandlung des hypoplastischen Linksherz-Syndroms

In contrast to other types of congenital heart defects, the treatment of hypoplastic left heart syndrome (HLHS) has become comparably successful only within the last decade. Postnatal management of circulatory disturbances of HLHS was previously often performed, similarly to other types of neonatal shock, without considering the peculiarities of postnatal hemodynamics. It is of overwhelming importance to limit pulmonary hyperperfusion by reducing systemic afterload and avoiding artificial respiration. The invention of selective hypothermic cerebral perfusion using the modified Blalock-Taussig shunt has decreased the need for long circulatory arrest times involving the brain, and promises a better neurological outcome. Postoperatively, sophisticated hemodynamic monitoring is mandatory to provide sufficient systemic oxygen delivery. α-blockers are usually given for strong afterload reduction. Hospital mortality is as low as 10–15% in centers experienced with the Norwood operation. The next surgical steps to create a serial systemic and pulmonary circulation involve superior cavopulmonary anastomosis performed as early as possible (4–6 months) and finally total cavopulmonary connection at an age of 3–4 years.     

The immunomodulatory effects of 3-monochloro-1,2-propanediol on murine splenocyte and peritoneal macrophage function in vitro.

3-Monochloro-1,2-propanediol (MCPD) is a well-known by-product of acid-hydrolyzed soy sauce during its manufacturing process. MCPD has been reported genotoxic in vitro, and reproductive toxicity and carcinogenicity in rats. To evaluate the immunomodulatory effect of MCPD on murine splenocyte and macrophage in vitro, we investigated splenocyte blastogenesis by concanavalin A (Con A), anti-CD3, and lipopolyssacharide (LPS), the production of cytokines from splenocyte, and the activity of mouse peritoneal macrophages. There was a significant decrease in lymphocyte blastogenesis to Con A or anti-CD3 at subtoxic dose of MCPD. A significant decrease in splenocyte blastogenesis to LPS was also observed. The production level of interferon (IFN)-gamma on splenocyte culture with Con A was significantly reduced at the higher concentration than 1.0mM of MCPD. The levels of interleukin (IL)-4 and IL-10 were also decreased at high concentrations of MCPD. There was a significant decrease in production of nitric oxide (NO) by peritoneal macrophages treated with MCPD. MCPD also inhibits tumor necrosis factor (TNF)-alpha production of stimulated macrophages. These results indicate that MCPD might be able to reduce the functionality of lymphocytes and peritoneal macrophages in vitro.     

Spontaneous resolution of prenatally diagnosed dural sinus thrombosis: a case report.

Fetal thrombosis of the dural sinus is an extremely rare congenital cerebrovascular condition that is potentially fatal. We report a case of dural sinus thrombosis diagnosed by prenatal ultrasonography and fetal magnetic resonance imaging (MRI) in the second trimester. The thrombosis showed partial resolution during pregnancy and resolved spontaneously after birth without neurological complications. This is the first report of spontaneous postnatal resolution, and may provide helpful information on the natural history and prenatal counseling of fetal thrombosis of the dural sinus.     

Phenotypic variability of a distinct deletion in McLeod syndrome

The X‐linked McLeod neuroacanthocytosis syndrome strongly resembles Huntington's disease and has been reported in various countries world‐wide. Herein, we report two Chilean brothers with predominant psychiatric features at disease onset including schizophrenia‐like psychosis and obsessive compulsive disorder. Molecular genetic analysis revealed a small deletion in the XK gene (938‐942delCTCTA), which has been already described in a North American patient of Anglo‐Saxon descent and a Japanese family, presenting with seizures, muscle atrophy or chorea yet absence of psychiatric features. These findings argue against a founder effect and indicate a profound phenotypic variability associated with the 938‐942delCTCTA deletion. Our report supports the inclusion of McLeod syndrome in the differential diagnosis of Huntington's disease as well as acute psychosis in male subjects. © 2007 Movement Disorder Society     

Clinical Significance of an Early Protocol Biopsy in Living-Donor Renal Transplantation: Ten-Year Experience at a Single Center

We report here our 10-year experience of a biopsy performed at day 14 after transplantation in 304 patients with stable graft function. The factors that may have influenced subclinical rejection were analyzed according to histology. The incidence of subclinical rejection was 13.2%. Addition of mycophenolate mofetile (MMF) as a primary immunosuppressant significantly decreased the incidence of subclinical rejection compared with patients without such treatment (odds ratio, 0.23; p < 0.05). On the other hand, HLA-DR antigen mismatch (odds ratio, 2.39) and unrelated donor (odds ratio, 2.10) were also significantly associated with decreased subclinical rejection (p < 0.05). The incidence of acute rejection in patients with normal findings was lower than in those with borderline changes or subclinical rejection (0.23 +/- 0.05 vs. 0.48 +/- 0.07 and 0.60 +/- 0.11, respectively; p < 0.05). The graft survival rates in patients with subclinical rejection were lower than in patients with normal or borderline changes at 1 (88.4% vs. 97.9% and 99.1%; p < 0.05), 5 (77.8% vs. 96.2% and 95.9%; p < 0.05) and 10 (62.3% vs. 96.2% and 93.7%; p < 0.05) years. Thus, a protocol biopsy performed on day 14 after transplantation is useful for predicting graft survival. Triple therapy including MMF, related donor and HLA-DR antigen match are important factors for reducing subclinical rejection in living-donor renal transplantation.     

Retinopathy in hepatitis C patients due to combination therapy with pegylated interferon and ribavirin

Background  

To assess the nature, incidence, and risk factors of retinopathy associated with pegylated interferon and ribavirin combination therapy in chronic hepatitis C patients.     

Thymidine labeling index in breast tumors

The relationship between primary tumor proliferative activity and clinical and pathologic characteristics was analyzed in relation to menopausal status in 32 patients with malignant or benign breast disease. The thymidine labeling index (TLI) showed significantly higher median values in the cancer patients (3.48 per cent) than in the patients with benign diseases (1.02 per cent). TLI was not significantly affected by delayed incubation at room temperature for about 1 hour. In the breast cancer patients, TLI did not significantly correlate to tumor size, the presence of axillary lymph node metastasis or pathologic nuclear grading. The only significant difference was limited to the breast cancer patients without axillary lymph node metastasis in relation to menopausal status; the TLI in the premenopausal patients (5.10 per cent) was significantly higher (p<0.05) than that in the postmenopausal patients (2.28 per cent). These data thus suggest that among premenopausal patients without axillary lymph node metastasis, those with a high TLI could be potential candidates for adjuvant chemotherapy.     

Synthesis and thermal properties of poly(arylene-1,2,4-oxadiazole)s with flexible,linear alkoxymethyl side branches

Poly(arylene-1,2,4-oxadiazole)s (PAOs) bearing flexible, linear side chains, 3a - c , were prepared by solution cyclodehydration of their precursors, poly(arylene acyloylamide oxime)s (PAAs), 2a - c , which were synthesized by solution polycondensation of 2,5-bis(n-alkoxymethyl)-terephthaldiamide oximes (DIS-TADO), 1a - c , with terephthaloyl dichloride (TPC). The length of the n-alkoxy group was varied from methoxy via butoxy to octyloxy. The reduction of the phase transition temperatures and the enhancement of the solubilities of 3a - c were greatly affected by the length of the side chains. PAOs with longer side chains, 3b and 3c , are nematic type liquid-crystalline polymers.